Alfie was born
Alfie was born on 10th June, weighing a healthy 8lbs 9oz.
8 Months Later
For the first 8 months of his life, Alfie seemed happy and healthy. Then one day we noticed that his right leg was swollen.
Visited the GP & Hospital
We took Alfie to our GP who referred us to Royal Aberdeen Children’s Hospital. X-rays revealed his right leg was longer than the other and that his spine was curved.
Our first vist to GOSH
Alfie had an MRI scan in December 2008 and as a result, we were sent to Great Ormond Street Hospital in London – a trip that changed our lives forever.
More than 600 miles from home, we finally received a diagnosis – Alfie had Lymphangiomatosis. The disease was in his right leg, pelvic area and marrow of certain bones. The condition also prevented his blood from clotting.
A frighful Journey
Alfie’s health deteriorated gradually and we embarked on the next phases of a journey which were distressing and fraught. It included one five-week period when Alfie suffered internal bleeding and frequently high temperatures and was subject to countless blood transfusions. At this point, we feared the worst as the doctors advised that if the bleeding did not stop there would be little hope for Alfie.
But, Alfie is a fighter. Within two days the bleeding stopped, his platelet count rose and his temperature came down.
Chemotherapy Treatment Started
Alfie started a treatment plan of Chemotherapy and Prednisolone, a type of steroid, to reduce the swelling in his leg. A week later he was stable enough to be moved to the hospital in Aberdeen for monitoring.
Alfie suffered severe nerve and bone pain within his leg and was put on to constant pain relief. He battled numerous unexplained high temperatures, infections and two fractured legs.
Heard about Sirolimus
We became aware of a drug called Sirolimus that was being given to children with Alfie’s condition. The long-term side effects were unknown but we had little choice but to try it.
He was weaned off the Vincristine and Prednisolone and started on the Sirolimus but with disastrous results – low platelets, internal bleeding and back into hospital for more blood transfusions. After three weeks the Sirolimus was stopped and we reverted to his old treatment plan.
Admitted to Hospital
This was a difficult year. Alfie was constantly admitted to hospital with high temperatures. At the end of the year, we discovered he had two fractured legs.
Hickman Line Infected
Alfie got an infection in his Hickman line. This had to be removed and was replaced with a port-a-cath.
Pain Relief Changed
The same treatment plan continued with very little improvement to his quality of life. In June the Doctors changed his pain relief from to Gabapentin as Alfie was suffering from side effects of the previously prescribed, Amytripiline.
Treatment Plan Change
In November his new Hickman line became infected. It was during this time the Doctors agreed that Alfie was probably the healthiest he had been in a long time and that they wanted to change his treatment plan again.
Prescribed Sirolimus Again
He was prescribed with Sirolimus in addition to his other medication – the combination instantly making a difference to his well-being. He was in less pain and was a much happier little boy.
Took His First Steps
Alfie took his first unaided steps aged 5. We hoped that his mobility would improve and he would be up walking and chasing his brother. This wasn’t the case as his continual growth caused more problems and became reliant on walking aids and a wheelchair.
We thought things were getting better. But Alfie then had a test to check his adrenal function – due to the length of time he had been on steroids. He failed this test miserably.
Alfie was now adrenal suppressant which means when he is unwell his body doesn’t produce the stress hormone cortisol which helps the body to cope. His medication was changed to accommodate this, we had to carry a hydro cortisol injection kit at all times – should he become unwell or experience any trauma.
Spleen Stopped Functioning
Alfie had another MRI scan, which showed that his spleen had reduced. The doctors believed that it was no longer functioning properly – resulting in Alfie being prescribed antibiotics on a long-term basis.
In and Out of Hospital
Every other month we seemed to be admitted back in the hospital with unexplained high temperatures. Our eldest son seemed to spend more and more nights at his Gran’s house so we could take Alfie into hospital. I soon learnt to have a bag packed ready to go.
Continued on Sirolimus for another 6 Months
At our final review of 2013 we were told that due to the lack of knowledge about the long-term side effects of Sirolimus, Alfie would be taken off the drug in the future. This medication has given Alfie some normality, allowing him to attend school and enjoy life. It was agreed that Alfie would remain on the drug for another six months.
Weaned Off Steroids
Life seemed to be settling down. Hospital admissions were becoming more infrequent as we managed to nurse him at home. We received positive news that his adrenal function was working and in November were able to wean him off the steroids.
Continues to Take Sirolimus
He continues on Sirolimus only experiencing mild side effects of the drug.
Clinically this has been his best year. Able to attend mainstream school and build relationships with friends, take up swimming and begin to be able to plan more for the future.
Over the years Lymphangiomatosis has caused problems with Alfie’s physical development.
Can No Longer Walk Unaided
The diseased leg is swollen, longer in length, tender to touch and now has a fixed knee and hip contracture causing the leg to be fixed at an angle. For short periods he can use a walking frame or crutches to get about. He has not walked unaided since 2012.
The Pyschological Impact of the Disease Starts to Show
Although things for us are getting easier, for Alfie they are becoming harder. He now knows more about his disease and the limitations that it has on his life. He will never be like his friends.
Informed of a Surgical Procedure
We learned about a surgical procedure that could be performed on Alfie’s leg to try and straighten it which may help him to walk again. Any operation on a child with Lymphangiomatosis comes with high risks of infection and lymphatic leakage – both life-threatening. However, we decided to go ahead with the procedure, which will take place in 2016.
Physiotherapy in Preparation for Surgery
We are preparing Alfie for surgery later in 2016. For this to go ahead the Alfie must be able to bear weight on his right leg. Alfie now does a stretching and physiotherapy programme every day with added cycling on his trike and swimming for fun.
Full Body MRI Scan
Alfie will also be given his first full-body MRI scan. He has had many scans but only of his leg and the pelvic area where the disease is present. Lymphangiomatosis can spread and can affect any part of the body – we hope there won’t be any surprises.
Planned operation to insert ‘eight plates’ and to release hamstrings in right leg – no complications with the operation but the procedure did not produce the outcome we had hoped for. After many months of physio and little improvement in his mobility, we have accepted that he is a full-time wheelchair user.
Full Body MRI Scan
First full body MRI showed areas of disease in his upper body – neck, chest, and collarbone.
Admission To Hospital
Admitted to hospital for a week with an extreme case of mouth ulcers
A Further Complication
Bouts of diarrhoea. Because of the low immune system, doctors diagnose bad bacteria in the gut.
This will be an ongoing problem that can be treated with medication.
More Planned Surgery
Planned surgery to have a new Port-a-cath inserted.
Review at GOSH
Review at Great Ormond Street Hospital. Alfie’s surgeon was disappointment that the surgical procedure didn’t give the outcome hoped for. A further full body MRI was done – the disease remains stable.
Admitted to hospital for 4 days with Cellulitis (bacterial infection) in his right foot.